Interstitial lung disease

What is interstitial lung disease?

Interstitial lung disease (ILD) refers to a group of more than 200 types of lung disorders that cause inflammation in the tissues surrounding the air sacs in your lungs. With each breath you take, tiny air sacs called alveoli expand and absorb oxygen. But in interstitial lung disease, the tissues that surround the air sacs are scarred and stiff, which makes it harder for them to expand and for your body to get enough oxygen. You’ll feel tired and have shortness of breath. Lung damage can’t be reversed, but therapies can help you breathe better, and treatments may stop the formation of new scars.

Penn Medicine’s pulmonology experts are leaders in using advanced technology to evaluate you and pinpoint the cause of your ILD. Specialists in Interstitial Lung Disease (ILD) Care meet with people who have ILD every day, so they can recognize patterns and have the expertise to diagnose even rare lung diseases. Our team includes pulmonologists, chest radiologists, and lung pathologists, among others, who work together to design an individualized treatment plan that can halt the damage to your lungs and make it easier for you to breathe.

Interstitial lung disease symptoms

Shortness of breath is the main symptom of interstitial lung disease. You may notice that you’re breathing harder or faster, or that you can only take shallow breaths. You might find that you breathe better when you lean forward while you’re sitting.

In addition to shortness of breath, you might also experience:

  • A dry cough that doesn’t produce mucus
  • Fatigue
  • Unexpected weight loss
  • Muscle and joint pain

Symptoms can be mild or severe and might only happen when you’re exercising or climbing stairs. As time goes on, you may have shortness of breath even when you’re not doing anything strenuous, like getting dressed. Eventually, you might feel out of breath when eating or talking. If ILD becomes advanced, your fingernails may become enlarged and curved, and your lips, skin, and nails may turn blue from low blood oxygen levels. You might also have symptoms of other diseases associated with ILD, like arthritis, high blood pressure, and heart problems.

Causes of interstitial lung disease

Autoimmune diseases like lupus, rheumatoid arthritis, sarcoidosis, and scleroderma can lead to stiffness of the tissues around air sacs, which can cause interstitial lung disease. You could also develop ILD if you had an infection like COVID-19 and didn’t fully recover. Also, some medicines like anti-inflammatory drugs, heart medicines, chemotherapy, radiation therapy, and others can cause lung damage that may lead to ILD. Anything you breathe in that causes inflammation can damage your lungs, including fungus, mold, asbestos, and dusts like coal dust, cotton dust, or silica dust. Smoking cigarettes also puts you at risk of ILD and can make ILD worse if you’ve already been diagnosed and continue to smoke.

Sometimes there’s no clear cause of your interstitial lung disorder. Most often, people with no known cause have idiopathic pulmonary fibrosis (IPF), which happens when scar tissue builds up in the lungs between the air sacs and blood stream. Medications have been approved to treat this type of ILD.

Diagnosing interstitial lung disease

During an evaluation for interstitial lung disease, your health-care provider listens to your lungs with a stethoscope. ILD causes dry, crackling breath sounds that your provider can hear during the exam. You may also get bloodwork to look for autoimmune diseases or rare lung diseases that may be causing your symptoms. High-resolution imaging scans may be taken to identify the location of your lung disease and how much it has progressed, which helps get you started on the most effective treatments as soon as possible.

Treating interstitial lung disease 

Care plans aim to slow the progression of ILD whenever possible and to treat any underlying causes of lung damage, like autoimmune disease. Medicines may be used to lower lung inflammation or slow the scarring of lung tissue. Certain therapies can teach you breathing methods, provide nutrition tips to boost your energy levels, and show how to set up your home to save energy. If you can, eliminate your exposure to substances that may be causing your symptoms, like stopping certain medications that can damage the lungs, quitting smoking, or removing mold or asbestos.

Our health-care providers do everything they can to help you breathe easier with medicines and pulmonary rehabilitation, though surgery may be recommended

Preventing interstitial lung disease

You might not be able to prevent ILD, but you can lower your risk by taking these steps:

  • If you smoke cigarettes, start a smoking cessation program.
  • Wear a respirator if your job requires working around asbestos, chemicals, or dusts.
  • Avoid or wear a respirator around allergens like mold.
  • Stay up to date on vaccines for pneumonia, flu, COVID-19, pertussis, and RSV.

Excellence in ILD care

If you have other conditions like autoimmune disease or pulmonary hypertension, we have a wide network of experts ready to provide you with care. If you need a lung transplant, our lung surgeons are national leaders in lung transplant care and have provided exceptional care to thousands of people who have needed a transplant.

We’ll support you through every step of your treatment journey. Our goal is to help you understand your diagnosis and treatment options, connect you with support groups and smoking cessation programs, and find other services that contribute to your physical and emotional healing as you work toward improving your lung function.

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